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Late congenital syphilis
See also in: Oral Mucosal Lesion
Other Resources UpToDate PubMed

Late congenital syphilis

See also in: Oral Mucosal Lesion
Contributors: Keshav Khanijow, Kenneth Katz MD, MSc, MSCE, Eric Ingerowski MD, FAAP
Other Resources UpToDate PubMed

Synopsis

Congenital syphilis is a congenitally acquired infection with the bacterium Treponema pallidum. Although it is a relatively uncommon presentation, congenital syphilis can be a devastating diagnosis, and earlier diagnosis carries the potential for significant improvement to an affected individual's quality of life. 

Transmission of T pallidum can occur in utero via the placenta after 4 months gestation, or through exposure to infectious vaginal lesions during delivery (perinatal syphilis). The subsequent signs and symptoms of congenital syphilis can be divided into two categories: (1) early congenital syphilis describes manifestations presenting before 2 years of age; (2) late congenital syphilis describes manifestations presenting after 2 years of age.

The symptoms of late congenital syphilis are analogous to tertiary syphilis in the adult. The signs and symptoms for both arise from either continued inflammatory processes against T pallidum antigens or attempts by the body to control the damage from infection in utero. Although late congenital syphilis is subclinical in 60% of cases, manifestations can occur into the early 20s and most commonly involve the bones, teeth, and nervous system. Hutchinson's triad, which is pathognomonic for late congenital syphilis, is defined by the presence of interstitial keratitis, Hutchinson incisors, and eighth nerve deafness.

Codes

ICD10CM:
A50.7 – Late congenital syphilis, unspecified

SNOMEDCT:
82323002 – Late congenital syphilis (2 years OR more)

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Various symptoms by themselves can be attributed to diseases other than late manifestations of congenital syphilis. However, multiple manifestations of late congenital syphilis increase the probability of diagnosis.
  • Many of the skeletal findings of late congenital syphilis overlap with those of rickets, including frontal bossing, Higouménakis sign, and saber shins.
  • In addition, in evaluating saber shins and Higouménakis sign, the possibility of underlying osteomyelitis, trauma, or tumors should be evaluated.
  • Saddle nose deformities can occur with trauma and infections.
  • Interstitial keratitis can occasionally occur with tuberculosis.
  • Serologic tests that are reactive for syphilis can also be attributed to other non-syphilis treponemal infections, or acquired syphilis.

Best Tests

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Therapy

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References

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Last Reviewed:10/13/2021
Last Updated:10/13/2021
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Late congenital syphilis
See also in: Oral Mucosal Lesion
A medical illustration showing key findings of Late congenital syphilis (General Manifestations) : Cranial nerve palsy, Deafness, High arched palate, Hutchinson teeth, Lip fissure, Maxillary hypoplasia, Saddle nose deformity, Skin erosions
Clinical image of Late congenital syphilis - imageId=1805532. Click to open in gallery.  caption: 'Hutchinson teeth (notched incisors).'
Hutchinson teeth (notched incisors).
Copyright © 2023 VisualDx®. All rights reserved.